Clinical use of ictal SPECT in secondarily generalized tonic-clonic seizures.

Partial seizures produce increased cerebral blood flow in the region of seizure onset. These regional cerebral blood flow increases can be detected by single photon emission computed tomography (ictal SPECT), providing a useful clinical tool for seizure localization. However, when partial seizures secondarily generalize, there are often questions of interpretation since propagation of seizures could produce ambiguous results. Ictal SPECT from secondarily generalized seizures has not been thoroughly investigated. We analysed ictal SPECT from 59 secondarily generalized tonic-clonic seizures obtained during epilepsy surgery evaluation in 53 patients. Ictal versus baseline interictal SPECT difference analysis was performed using ISAS (http://spect.yale.edu). SPECT injection times were classified based on video/EEG review as either pre-generalization, during generalization or in the immediate post-ictal period. We found that in the pre-generalization and generalization phases, ictal SPECT showed significantly more regions of cerebral blood flow increases than in partial seizures without secondary generalization. This made identification of a single unambiguous region of seizure onset impossible 50% of the time with ictal SPECT in secondarily generalized seizures. However, cerebral blood flow increases on ictal SPECT correctly identified the hemisphere (left versus right) of seizure onset in 84% of cases. In addition, when a single unambiguous region of cerebral blood flow increase was seen on ictal SPECT, this was the correct localization 80% of the time. In agreement with findings from partial seizures without secondary generalization, cerebral blood flow increases in the post-ictal period and cerebral blood flow decreases during or following seizures were not useful for localizing seizure onset. Interestingly, however, cerebral blood flow hypoperfusion during the generalization phase (but not pre-generalization) was greater on the side opposite to seizure onset in 90% of patients. These findings suggest that, with appropriate cautious interpretation, ictal SPECT in secondarily generalized seizures can help localize the region of seizure onset.

Cortical and subcortical networks in human secondarily generalized tonic-clonic seizures.

Generalized tonic-clonic seizures are among the most dramatic physiological events in the nervous system. The brain regions involved during partial seizures with secondary generalization have not been thoroughly investigated in humans. We used single photon emission computed tomography (SPECT) to image cerebral blood flow (CBF) changes in 59 secondarily generalized seizures from 53 patients. Images were analysed using statistical parametric mapping to detect cortical and subcortical regions most commonly affected in three different time periods: (i) during the partial seizure phase prior to generalization; (ii) during the generalization period; and (iii) post-ictally. We found that in the pre-generalization period, there were focal CBF increases in the temporal lobe on group analysis, reflecting the most common region of partial seizure onset. During generalization, individual patients had focal CBF increases in variable regions of the cerebral cortex. Group analysis during generalization revealed that the most consistent increase occurred in the superior medial cerebellum, thalamus and basal ganglia. Post-ictally, there was a marked progressive CBF increase in the cerebellum which spread to involve the bilateral lateral cerebellar hemispheres, as well as CBF increases in the midbrain and basal ganglia. CBF decreases were seen in the fronto-parietal association cortex, precuneus and cingulate gyrus during and following seizures, similar to the 'default mode' regions reported previously to show decreased activity in seizures and in normal behavioural tasks. Analysis of patient behaviour during and following seizures showed impaired consciousness at the time of SPECT tracer injections. Correlation analysis across patients demonstrated that cerebellar CBF increases were related to increases in the upper brainstem and thalamus, and to decreases in the fronto-parietal association cortex. These results reveal a network of cortical and subcortical structures that are most consistently involved in secondarily generalized tonic-clonic seizures. Abnormal increased activity in subcortical structures (cerebellum, basal ganglia, brainstem and thalamus), along with decreased activity in the association cortex may be crucial for motor manifestations and for impaired consciousness in tonic-clonic seizures. Understanding the networks involved in generalized tonic-clonic seizures can provide insights into mechanisms of behavioural changes, and may elucidate targets for improved therapies.

Treatment of presumed arrhythmogenic right ventricular dysplasia in an adolescent.

Familial arrhythmogenic right ventricular dysplasia is a rare cardiomyopathy that is usually diagnosed on postmortem examination or on presentation with progressive congestive heart failure. We present a patient in whom an automatic implantable cardioverter-defibrillator was inserted prophylactically. A review of the condition and possible therapies is included.

A five-year clinical experience with 112 Blalock-Taussig shunts.

In spite of recent advances in neonatal open repair for complex cyanotic heart disease, some patients require palliation with a systemic-to-pulmonary artery shunt. We report a 5-year experience (1985-1990) with 112 Blalock-Taussig shunts. Forty-six of the 92 patients had some variant of tetralogy of Fallot, with a wide spectrum of diagnoses in the remainder. The median age at surgery was 3 months. A classic Blalock-Taussig shunt was done in 26% (group I), and a 4- or 5-mm PTFE graft was utilized in the remainder (group II). The technical aspects of each of the procedures are reviewed. There were three early deaths in the entire group, none of them related to Blalock-Taussig shunt function. There was no incidence of early shunt insufficiency, bleeding, infection, limb ischemia, or pulmonary artery distortion. There was a 21% incidence of clinical congestive heart failure, seen somewhat more commonly in group I. The overall need for reshunting/open repair was similar in both groups, but there was a statistically longer interval between the initial Blalock-Taussig shunt and the second procedure in group I (21.6 vs 12.4 months). The Blalock-Taussig shunt remains a safe, reliable, and effective means of increasing pulmonary flow.

Congenital aortic regurgitation: natural history and management.

OBJECTIVES AND BACKGROUND: Congenital aortic regurgitation is rare as an isolated lesion. We describe seven children with no physical features of the Marfan syndrome in the patients or their families and no other cardiac lesions who had congenital valvular aortic regurgitation. METHODS: From 1954 to the present, seven children with auscultatory and physiologic characteristics of aortic regurgitation were evaluated for a total of 108 patient-years. We report on their natural history, clinical and laboratory findings, management and outcome. RESULTS: In five of the seven children congenital aortic regurgitation was diagnosed in infancy. In four, progressive severity of the regurgitation led to valve replacement at age 3, 10, 15 and 20 years, respectively, and to resection of an aneurysm of the ascending aorta in the 10-year old patient. Two patients had cystic medial necrosis on aortic biopsy. One of these patients died after reoperation for dissecting aneurysm of the thoracic aorta at 22 years of age; the other died after dissection and rupture of the ascending aorta at age 25 years. After obstructing pannus developed, the 3-year old patient underwent replacement of the St. Jude valve at age 10 years. The other three patients were asymptomatic at last follow-up at age 8, 10 and 20 years, respectively. CONCLUSIONS: Supportive management is recommended until it becomes necessary to intervene surgically when regurgitation becomes severe. The need for surgical treatment is indicated by the appearance of a diastolic thrill, left ventricular strain on the electrocardiogram or other evidence of left ventricular dysfunction on the echocardiogram or exercise stress testing by treadmill or radionuclide cineangiocardiography. Close follow-up of these patients is important to detect progression of aortic regurgitation, especially in the presence of cystic medial necrosis.

Transient complete heart block following percutaneous balloon pulmonary valvuloplasty: treatment with systemic corticosteroids.

Balloon pulmonary valvuloplasty is a safe and effective treatment for congenital pulmonic valve stenosis. This report describes a child who developed complete atrioventricular (AV) block following balloon pulmonary valvuloplasty. The child was treated with a 10-day course of systemic corticosteroids. The heart block gradually resolved during the first week following the procedure and has not recurred. Heart block is a known complication of right heart catheterization and has been described following balloon pulmonary valvuloplasty. Systemic corticosteroids have been used to treat AV block of various etiologies and may have contributed to the child's recovery in this case.

Epidemiologic aspects of Kawasaki disease in a Manhattan hospital.

Epidemiologic and clinical features of Kawasaki disease in 106 patients seen between 1980 and 1986 at The New York Hospital in midtown Manhattan were compared with those in large series from the United States, Canada, and Japan. Dissimilarities in our Kawasaki disease experience included ethnic heterogeneity of our patients (50% white, 18% black, 16% Hispanic, and 16% Oriental) and, in comparison with the Japanese experience, an older mean age (3 1/2 vs 1 1/2 years) with fewer children less than 2 years of age (32% vs 50% to 60%). In comparison with the general population of the geographic urban and suburban referral area for our hospital and in comparison with our general pediatric population, Oriental children with Kawasaki disease were overrepresented (16% vs 2%). More families of children with Kawasaki disease were members of the upper and middle class (73%) than were the population seen in general pediatrics (31.7%) at our hospital. Personal interviews with 63 families of children with Kawasaki disease and 63 control families with children paired for ethnic group, sex, and age revealed no epidemiologic differences except for use of rug shampoo within 1 month of onset in 16 episodes in 15 children with Kawasaki disease in 14 families (22% of families) compared with two families of control children (3%) (P less than .001).

Coronary artery involvement in Kawasaki syndrome in Manhattan, New York: risk factors and role of aspirin.

Since January 1980, 110 children having 113 attacks of Kawasaki syndrome were studied. Age at onset was 7 weeks to 12 years (mean 3 6/12 years, median 2 9/12 years); 77% were younger than 5 years of age; the male to female ratio was 1.8; racial distribution was 52% white, 19% black, 14% Hispanic, and 16% Asian. Protocol of management consisted of high-dose aspirin (100 mg/kg/d) until afebrile, and then 81 mg every day until free of coronary aneurysm. Two-dimensional echocardiograms were done weekly during the acute stage, at 2 and 6 months after onset, and yearly if a coronary abnormality was detected. At 1 month, 51 coronary arterial abnormalities were present in 25 patients. Risk factors for a coronary abnormality were duration of fever greater than or equal to 2 weeks, level of platelet count, marked elevation of ESR, and age younger than 5 years. No statistically significant difference in incidence of aneurysms was detected between patients on high-dose aspirin and those on medium-or low-dose aspirin.

Aortic aneurysm secondary to umbilical artery catheterization.

A 14-month-girl presented with an asymptomatic posterior mediastinal mass. She had a history of prematurity, umbilical artery catheterization, and sepsis. The diagnosis of aortic aneurysm was made by dynamic computed tomography. The aneurysm was successfully resected.

Determinants and importance of atrial pressure morphology in atrial septal defect.

A prominent "v" wave relative to the "a" wave in the jugular vein and right atrial pressure tracing is considered to be a common haemodynamic sign of atrial septal defect. Since the prevalence, age relation, and haemodynamic determinants of the "v" greater than or equal to "a" wave configuration have not been studied the pressure recordings from 15 adults and 80 children with an isolated secundum atrial septal defect in sinus rhythm and from 40 adults and 55 children in sinus rhythm without structural cardiac abnormalities or with coronary and valvular heart disease were studied to assess the sensitivity and specificity of the "v" greater than or equal to "a" wave configuration in atrial septal defect. Only 20% of adults with an atrial septal defect had prominent right atrial "v" waves compared with 63% of children, although the specificity was quite high for each group. In adults "left atrialisation " of the right atrium ("v" greater than or equal to "a" wave) occurred in younger patients with higher right atrial and right ventricular end diastolic pressures. In contrast, in children no age related or haemodynamic determinants for the "v" greater than or equal to "a" pattern were found. In addition, most adults but few children with an atrial septal defect had "right atrialisation " of the left atrial wave configuration ("a" greater than "v"). This was found in older adults with lower right atrial and right ventricular end diastolic pressures and in older children with larger left to right shunts. Thus in contrast to children adults with an atrial septal defect rarely show a prominent "v" wave in the right atrium. The presence of a prominent right atrial "v" wave in adults with an atrial septal defect is associated with relatively higher left atrial and right heart pressures than is the absence of this sign and may be related to relatively higher systolic transatrial flow in these patients. The relative paucity of prominent right atrial "v" waves in older adults suggest that the systolic phase flow may diminish with age, possibly from progressive alteration in compliance of the chronically dilated right ventricle.

Digital angiography in the pediatric patient with congenital heart disease: comparison with standard methods.

Digital subtraction angiography (DSA) permits high-resolution cardiac imaging with relatively low doses of contrast medium and reduced radiation exposure. These are potential advantages in children with congenital heart disease. Computer-based DSA (30 frames/sec) and conventional cutfilm angiography (6 frames/sec) or cineangiography (60 frames/sec) were compared in 42 patients, ages 2 months to 18 years (mean 7.8 years) and weighing 3.4 to 78.5 kg (mean 28.2 kg). There were 29 diagnoses that included valvular regurgitant lesions, obstructive lesions, various shunt abnormalities, and a group of miscellaneous anomalies. For injections made at a site distant from the lesion and on the right side of the circulation, the mean dose of contrast medium was 60% to 100% of the conventional dose given during standard angiography. With injections made close to the lesion and on the left side of the circulation, the mean dose of contrast medium was 27.5% to 42% of the conventional dose. Radiation exposure for each technique was markedly reduced in all age groups. A total of 92 digital subtraction angiograms were performed. Five studies were suboptimal because too little contrast medium was injected; in the remaining 87 injections, DSA and conventional studies resulted in identical diagnoses in 81 instances (p less than .001 vs chance). The remaining six injections made during DSA failed to confirm diagnoses made angiographically by standard cutfilm angiography or cineangiography. We conclude that DSA usually provides diagnostic information equivalent to that available from cutfilm angiography and cineangiography, but DSA requires considerably lower doses of contrast medium and less radiation exposure than standard conventional methods.

Acute effects of vasodilators on left-to-right shunts in infants and children.

Based on the favorable experience with vasodilator therapy in adult heart disease and the results of acute dogs experiments, we undertook the hemodynamic evaluation of hydralazine and phentolamine during diagnostic cardiac catheterization. We studied seven infants and children with left-to-right (leads to R) shunts at atrial, ventricular, or ductal levels to determine whether vasodilator therapy might be useful in the treatment of infants with congestive heart failure (CHF) due to large L leads to R shunts. Shunts, flows, and resistances were measured by the indicator dilution and Fick techniques before and after administration of the drug. At a dose sufficient to produce an effect, the shunt flow increased after each drug. There is no evidence from studies at cardiac catheterization of therapeutic efficacy for vasodilators in the treatment of CHF due to cardiac L leads to R shunts.